Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Inflammatory bowel diseases are a result of an aberrant mucosal immune response to gut microflora. Several groups have reported newly diagnosed inflammatory bowel diseases following solid organ transplantation and subsequent immunosuppressive therapy. We describe four cases of newly diagnosed inflammatory bowel diseases following liver transplantation in a pool of 120 transplanted patients. These patients had no prior history of inflammatory bowel diseases or primary sclerosing cholangitis and were immunosuppressed. Two patients were transplanted for a hepatitis C related cirrhosis, one for alcoholic cirrhosis and one patient for autoimmune cirrhosis. Three patients were diagnosed with ulcerative colitis and one with Crohn's disease. These four patients were on a cyclosporin monotherapy when their inflammatory bowel diseases were diagnosed. These data suggest that cyclosporin monotherapy following solid organ transplantation does not prevent development of inflammatory bowel diseases.
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PMID:[De novo inflammatory bowel diseases after liver transplantation: description of four new cases and a review of the literature]. 1707 59

Recently, the incidence of intrahepatic cholangiocarcinoma (ICC) has been increasing in a number of developed (Western) countries. However, risk factors in these low-risk populations are poorly understood. In this nationwide population based case-control study in Denmark, we examined the relationship between selected medical conditions and subsequent ICC risk to provide additional clues to etiopathogenesis. All histologically confirmed ICC cases diagnosed in Denmark between 1978 and 1991 were identified from the Danish cancer registry. Population controls were selected from the central population registry and were matched 4:1 to cases on sex and year of birth. Cases and controls were linked to the Danish hospital discharge registry to obtain information on prior hospital diagnoses. Odds ratios (OR) and 95% confidence intervals (95% CI) were derived using conditional logistic regression. A total of 764 ICC cases and 3,056 population controls were included in the study. Chronic liver diseases were significantly related to ICC: alcoholic liver disease (OR = 19.22, 95% CI = 5.55-66.54), unspecified cirrhosis (OR = 75.9, 95% CI 10.2-565.7). Bile duct diseases were also associated with risk: cholangitis (OR = 6.3, 95% CI = 2.3-17.5), choledocholithiasis (OR = 23.97, 95% CI = 2.9-198.9), cholecystolithiasis (OR = 4.0, 95% CI = 2.0-7.99), though gallbladder removal did not change risk (OR = 1.6, 95% CI = 0.65-3.7). Among other conditions, chronic inflammatory bowel disease (OR = 4.7, 95% CI = 1.65-13.9) was significantly associated with ICC. Diabetes was associated with risk in the year prior to diagnosis of ICC (OR = 3.02, 95% CI = 1.05-8.69). Obesity was unrelated to risk. These results confirm that prior bile duct diseases increase risk of ICC and suggest that alcoholic liver disease and diabetes may also increase risk.
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PMID:Risk factors for intrahepatic cholangiocarcinoma in a low-risk population: a nationwide case-control study. 1710 84

Primary sclerosing cholangitis is a chronic cholestatic liver disease of unknown origin characterized by progressive inflammation, destruction, and fibrosis of the intrahepatic and extrahepatic bile ducts. The disease leads to obliteration of intrahepatic bile ducts and to biliary cirrhosis, end-stage liver disease, and portal hypertension. Primary sclerosing cholangitis commonly occurs in the presence of inflammatory bowel disease. Its exact etiology remains unknown. As a result, there is no existing effective medical management to delay or modify the progression of the disease. Ursodeoxycholic acid, the most well-studied drug for primary sclerosing cholangitis, has demonstrated promising results when used in combination with an immunosuppressant or antibiotic. To date, liver transplantation remains the only confirmed long-term treatment of primary sclerosing cholangitis, which now accounts for 6% of adult and 1% of pediatric liver transplantations in the United States. Primary sclerosing cholangitis represents an important liver disease with major morbidity and mortality.
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PMID:Primary sclerosing cholangitis. 1744 Mar 11

The plasma metalloprotease ADAMTS13 (A Disintegrin And Metalloprotease with ThromboSpondin type 1 motif 13) cleaves prothrombotic ultralarge multimers of the platelet-adhesive protein von Willebrand factor (ULVWF) into less active multimers that promote haemostasis in injured blood vessels. When the enzyme is dysfunctional or undetectable, circulating ULVWF may cause massive intravascular aggregation of platelets and thrombotic thrombocytopenic purpura. This study compared ADAMTS13 antigen and activity in a large set of plasmas collected from subjects with various conditions of health and disease, most of which were associated with an increased thrombotic tendency. Pathological conditions were liver cirrhosis (n = 90), inflammatory bowel disease (n = 44) and cardiac surgery (n = 30). Healthy conditions were pregnancy (n = 42), oral contraceptive intake (n = 33) and the neonatal state (n = 41). Normal individuals of different ages were taken as controls (n = 132). The antigen assay showed less variability than the collagen binding-based activity assay. Antigen values correlated well with activity in normal individuals, but were discrepant to various degrees in neonates, pregnancies of later maternal age and cardiac surgery. No discrepancies were noted in liver cirrhosis and inflammatory bowel disease, which were both associated with low-plasma levels of ADAMTS13. The parallel measurement of ADAMTS13 activity and antigen provides a new tool for understanding the behaviour of the VWF cleaving protease in health and disease.
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PMID:ADAMTS13 activity to antigen ratio in physiological and pathological conditions associated with an increased risk of thrombosis. 1760 62

Cholangiocarcinoma occurs with a varying frequency in different areas of the world. Some of the variations in incidence rates can be explained by the distribution of risk factors in different geographic regions and ethnic groups. Several accepted risk factors for cholangiocarcinoma include infestation with liver flukes, primary sclerosing cholangitis, hepatolithiasis, choledochal cysts, cirrhosis, and infusion of certain chemical agents. Approximately, 90% of patients diagnosed with cholangiocarcinoma do not have a recognized risk factor for the malignancy. The study by Ahrens et al. [16] finds that obesity and gallstones are risk factors for developing extrahepatic cholangiocarcinoma in men patients. Obesity was found to have a 'dose-effect' relationship with the strength of statistical association. No significant association was reported for tobacco or alcohol use, hepatitis, cirrhosis, diabetes, or inflammatory bowel disease. Although the author's definition of extrahepatic cholangiocarcinoma was unusual, the association of obesity with the risk of developing cholangiocarcinoma persisted for all anatomic subsites.
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PMID:Risk factors for cholangiocarcinoma. 1762 30

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown etiology that is frequently associated with inflammatory bowel disease. It is characterized by diffuse inflammation and fibrosis of the biliary tree, and it usually leads to biliary cirrhosis and portal hypertension. PSC is most commonly diagnosed with endoscopic retrograde cholangiopancreatography, although magnetic resonance cholangiography (MRC) is rapidly emerging as a first choicediagnostic test. MRC has the advantage of being non-invasive, does not require radiation, and is cost-effective in that it does not carry the risk of pancreatitis associated with retrograde studies.(2) Percutaneous cholangiography is seldom performed anymore.
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PMID:Characteristics of primary sclerosing cholangitis in the USA. 1793 Dec 6

The authors present the results of a long-term monitoring of patients who presented with clinical signs of bleeding from the upper gastrointestinal tract (GIT) but totally negative endoscopy results on admission. Retrospective-prospective analysis of acute endoscopies in patients with clinical signs of bleeding from the upper gastrointestinal tract performed in the Endoscopy Centre of the OstravaTeaching Hospital from 2002 to 2005, long term monitoring of the sample with negative results on admission. A total of 133 patients, i.e. 16.3 % of all acute procedures, with no source of bleeding detected in the first endoscopy. 26.4% of wrong indications. In the rest of the sample, a source of bleeding was detected by endoscopy in 17 patients (15 in an early examination and 2 within one month from the event), i.e. 17.3%. The rate of bleeding recurrence was 15%. The risk of recurrence was higher in patients with haematemesis and a decrease in the blood count (BC) at the time of the first event. In 19 patients, i.e. 14.3%, the source was not detected. In the remaining 33 patients, the findings were as follows: 1) in 4 patients, the source of bleeding was detected in the small intestine (push enteroscopy or enteroclysis); 2) in 3 patients, the source of bleeding was detected in the colon; 3) in 6 patients, IBD was detected; 4) in 5 patients, cirrhosis of the liver was diagnosed within months or years from the event.
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PMID:[Long-term monitoring of patients with clinical symptoms of upper gastrointestinal tract bleeding with a negative endoscopy result]. 1801 62

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease, characterized by progressive inflammation and fibrosis of the bile ducts, resulting in biliary cirrhosis and is associated with a high risk of cholangiocarcinoma. The majority of patients are young, male and have coexisting inflammatory bowel disease. PSC is found with a prevalence of 10/100,000 in Northern European populations. The pathophysiology of PSC is a complex multistep process including immunological mechanisms, immunogenetic susceptibility and disorders of the biliary epithelia. The diagnosis is primarily based on endoscopic cholangiography although magnetic resonance imaging is increasingly used; biochemistry and immunoserology as well as histology play only a minor role. Due to the high risk of developing cholangiocarcinoma and also other tumours of the GI tract, surveillance strategies are essential, however they have yet to be established and evaluated. Biochemical parameters, clinical risk factors, endoscopic procedures and imaging techniques contribute to the early identification of patients at risk. Since medical therapy of PSC with ursodeoxycholic acid does not improve survival, to date, liver transplantation is the only option with a cure potential; if transplantation is accurately timed, transplanted PSC patients have an excellent rate of survival. However if cholangiocarcinoma is detected, a curative treatment is not possible in the majority of cases. The present review critically summarizes the current knowledge on the aetiopathogenesis of PSC and gives an overview of the diagnostic approaches, surveillance strategies and therapeutic options. Primary sclerosing cholangitis is a disease of unknown aetiology and without any further curative treatment options apart from liver transplantation. Therefore it may be regarded as the greatest challenge in hepatology today.
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PMID:The challenges in primary sclerosing cholangitis--aetiopathogenesis, autoimmunity, management and malignancy. 1876 Aug 56

Primary sclerosing cholangitis (PSC) is a chronic, cholestatic liver condition characterized by progressive fibrosis and destruction of the intra- and extrahepatic biliary tree. PSC has a clear association with inflammatory bowel disease and is often progressive, leading to cirrhosis and end-stage liver failure. For many patients, liver transplantation offers the only hope of long-term survival. No effective medical treatment exists, and therapy is often aimed at treating complications of the disorder, including dominant biliary strictures, which may cause symptomatic jaundice, cholangitis, and pruritus. Studies on endoscopic therapy (eg, biliary dilation and/or stent insertion) have shown favorable results, although most studies have been small, retrospective, and uncontrolled. Up to 20% of patients with PSC develop cholangiocarcinoma; however, distinguishing between cholangiocarcinoma and benign strictures can be difficult. Ideally, randomized trials are required to determine the safest and most effective endoscopic management for symptomatic dominant strictures.
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PMID:Endoscopy in the management of primary sclerosing cholangitis. 1846 5

Primary sclerosing cholangitis is a chronic cholestatic liver disease characterized by inflammation and fibrosis of the bile ducts, resulting in cirrhosis and need for liver transplantation and reduced life expectancy. The majority of cases occur in young and middle-aged men, often in association with inflammatory bowel disease. The etiology of primary sclerosing cholangitis includes immune-mediated components and elements of undefined nature. No effective medical therapy has been identified. The multiple complications of primary sclerosing cholangitis include metabolic bone disease, dominant strictures, bacterial cholangitis, and malignancy, particularly cholangiocarcinoma, which is the most lethal complication of primary sclerosing cholangitis. Liver transplantation is currently the only life-extending therapeutic alternative for patients with end-stage disease, although recurrence in the allografted liver has been described. A PSC-like variant attracting attention is cholangitis marked by raised levels of the immunoglobulin G4 subclass, prominence of plasma cells within the lesions, and steroid responsiveness.
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PMID:Clinical features and management of primary sclerosing cholangitis. 1852 31


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